Clearing house for health, nursing and aged care issues in Australia. Along with help for those studying to become a Health Professional

What is Fibrous dysplasia

Fibrous dysplasia is a condition that causes abnormal growth or swelling of bone. The affected bone becomes enlarged, brittle and warped. Fibrous dysplasia can occur in any part of the skeleton but the bones of the skull and face, thigh, shin, ribs, upper arm and pelvis are most commonly affected.

This rare disorder is usually diagnosed in childhood or early adulthood and can affect one or several bones. Males and females of any race are equally affected.

Fibrous dysplasia is not a form of cancer and does not increase a person’s susceptibility to cancer. In some cases, hormone problems and changes in skin colour also occur. Fibrous dysplasia is incurable but can be managed with medical treatment.

Symptoms
In some cases, fibrous dysplasia has no symptoms and is only diagnosed by accident during investigations for an unrelated medical problem. When symptoms occur, they can include:

* Unusual gait – for example, ‘waddling’ or rocking from side to side when walking
* Pain – caused by the expansion of bone or the pressure of the expanding bone against a nerve
* Irregular bone growth
* Bone deformity
* Increased susceptibility to bone fractures.

Possible complications
Fibrous dysplasia can cause a range of complications:

* Rickets may develop in some cases.
* Affected pelvis and leg bones increase the risk of arthritis developing in the hip and knee joints.
* Eyesight problems or other sensory problems may develop if the bones of the skull are affected.

Different types of fibrous dysplasia
The main types of fibrous dysplasia include:

* Monostotic fibrous dysplasia – only one bone is affected. This accounts for about seven cases out of every 10. The most commonly affected site in monostotic fibrous dysplasia is the skull.
* Polyostotic fibrous dysplasia – two or more bones are affected.
* McCune-Albright syndrome – fibrous dysplasia can be associated with hormone disturbances and skin pigment changes. About one case of fibrous dysplasia in every 10 is diagnosed as McCune-Albright syndrome.

McCune-Albright syndrome
Some people with fibrous dysplasia have hormone problems and changes in skin colour. This is known as McCune-Albright syndrome. Generally, people who have fibrous dysplasia in more than one bone are at increased risk of developing McCune-Albright syndrome. Symptoms may include:

* Onset of puberty before 10 years of age
* Overactive pituitary gland, which could lead to abnormal height
* Overactive thyroid gland (hyperthyroidism)
* Overactive parathyroid gland, which leads to excessive levels of blood calcium and can cause persistent nausea and vomiting
* Darkened spots on the skin (café-au-lait spots).

The cause of fibrous dysplasia is unknown
The cause seems to be a genetic mutation that alters the normal growth of the bone’s connective tissue. It is not clear whether the gene responsible for the condition is inherited. Some researchers believe that the gene mutates after conception while the baby is developing in the womb. The possible causes of the mutation are not known.

Diagnosis methods
Sometimes, the symptoms of fibrous dysplasia mimic the symptoms of other conditions. For example, fibrous dysplasia of the vertebrae (backbones) may be misdiagnosed as idiopathic scoliosis. Fibrous dysplasia is diagnosed using a number of tests, including:

* Treatment to reduce the risk of complications such as rickets or fractures
* Medication to strengthen bones (such as drugs commonly used in the treatment of osteoporosis)
* Medication to treat pain, hormone imbalances and other problems
* Pain management therapy
* Physiotherapy to improve joint mobility
* Surgery to correct bone deformities.

Surgery options
Surgery is recommended in the case of painful or fractured bones or when joints can no longer move freely. Fibrous dysplasia that causes complications such as pressure against the brain, spinal cord or nerves is also treated with surgery.

Usually, the section of diseased bone is removed. The remaining bone is strengthened with grafts of healthy bone tissue taken from other unaffected areas of the skeleton. In some cases, pins, rods and other permanent devices may be inserted to further bolster the bone.

Surgery may successfully treat the condition if only one bone is affected. If several bones are affected, it may not be possible to remove every section of diseased tissue.

Exercise is important
Regular weight bearing exercise helps to strengthen bone, increase joint mobility and maintain a healthy weight. It is important that the exercise program is performed under the supervision of a doctor because a person with fibrous dysplasia is at increased risk of bone fractures.

* Fibrous dysplasia is a condition that causes abnormal growth or swelling of bone.
* The cause seems to be a genetic mutation that alters the normal growth of the bone’s connective tissue.
* Treatment includes surgery to remove diseased section of bone.

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5 Responses

Hi I have puzzled doctors for yrs even after a biopsy which revealed that it was neither F.D. or Paget’s disease, I the 2 Dr.s that reported it wrong. It is located in my right temple area and is now seriously causing deafness and my sight is terrible. I am 60 but out side of that I am very healthy. My balance is terrible and experience head PAIN !!!. My neck hurts all the time and have a large skull etc. etc.. Not really sure what to do, my right and is lower than my left there is spot in my sinus etc.. Just thought I would write this and no it doesn’t make it any less uncomfortable. Don’t have a Dr., it seems they have more interest dianosing it than treating it. Respectfully richard

Hi Richard, I feel for you Richard as I too have puzzeled Dr for the last 33 years. I think I have FD of the spine (T3) at least that is what the CT scan sugested and the biopsy says is consistent with.The Dr just sent me a letter (I live in the country)and said “good news, have another scan in 12 mounths” Thank goodness for the internet or I wouldn’t even know what FD was.

There is a lot more to FD than what is printed here. I have a son that has MAS and what is written here is really sugar coated. There are a lot more complications than what they list. As far as for plastic surgery of the skul – I highly recomend not having any type of surgery. I have seen results of plastic surgery of people with FD – and they are not positive. More info can be found at my son’s website – http://www.jakesjourney.org – good luck to anyone with this and other diseases. I know it is a long hard road.

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